Rheumatology Medical News about ANCA-associated vasculitides;nervous system involvement

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Clinical analysis of nervous system involvement in ANCA-associated systemic vasculitides
Clinical and Experimental Rheumatology Online, 09/08/09

Zhang W et al. – NS involvement was common in AAVs and the characteristic of NS involvement was different among MPA, WG and CSS patients. DAH and infection instead of NS damage remained the leading causes of death in AAVs.

Methods

179 patients, including 93 cases of MPA, 61 cases of WG, and 25 cases of CSS, were enrolled in this study.

Results

NS involvement wasobserved in 36.6% of MPA, 50.8% of WG, and 76.0% of CSS patients
Peripheral neuropathy predominated in each type of AAV
In CSS and MPA, the majority was mononeuritis multiplex and distal symmetrical polyneuropathy, whereas, 64.5% of WG patients with NS involvement had cranial neuropathy
CNS involvement accounted for 21.1%, 29.4%, and 32.3% of neuropathy respectively in CSS, MPA, and WG patients, respectively, including arachnoid hemorrhage, cerebrovascular neuro-pathy, meningitis, and diffuse brain damage
157 (87.7%) AAV patients responded to treatment with high dose of prednisone plus immunosuppressants
Thirteen (14.0%) MPA and four (6.6%) WG patients died
The leading causes of death were diffuse alveolar hemorrhage (DAH) (6, 35.3%) and infection (6, 35.3%)
No patient died directly of neuropathy

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